Fumarase/FH Mouse mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss
KO/KD Validated
货号：bsm-33167M
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概述
产品编号
bsm-33167M
产品类型
KO/KD Validated
英文名称
Fumarase/FH Mouse mAb
中文名称
富马酸水合酶单克隆抗体
英文别名
FMRD; HLRCC; HsFH; LRCC; MCL; MCUL1; Fh; Fh-1; Fumarase; Fh1; im:7152785; ns:zf-e152; zf-e152; zgc:66253; zgc:77498; FUMH_HUMAN; 4.2.1.2; FUMH_MOUSE; EF-3; FUMH_PIG; FUMH_RAT; FUMH_DANRE; fumarate hydratase
抗体来源
Mouse
免疫原
Recombinant human Fumarase/FH Protein
亚型
IgG
性状
Size : 50ul/100ul/200ul
Liquid
Size : 200ug (PBS only)
Lyophilized
Note: Centrifuge tubes before opening. Reconstitute the lyophilized product in distilled water. Optimal concentration should be determined by the end user.
Liquid
Size : 200ug (PBS only)
Lyophilized
Note: Centrifuge tubes before opening. Reconstitute the lyophilized product in distilled water. Optimal concentration should be determined by the end user.
纯化方法
affinity purified by Protein G
克隆类型
Monoclonal
克隆号
4C12
理论分子量
51 kDa
检测分子量
42 kDa
浓度
1mg/ml
储存液
Size : 50ul/100ul/200ul
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Size : 200ug (PBS only)
0.01M PBS
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Size : 200ug (PBS only)
0.01M PBS
研究领域
Cancer > Cancer Metabolism > Metabolic signaling pathway > Integration of energy metabolism
Cancer > Oncoproteins/suppressors > Tumor suppressors
Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Energy Metabolism
Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Integration of energy
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008]
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| WB | Human | Mouse, Rat | 1:500-3000 |
| IHC-P | Human, Mouse, Rat | 1:100-500 | |
| IHC-F | Human, Mouse, Rat | 1:100-500 | |
| IF | Human, Mouse, Rat | 1:100-500 |
交叉反应
交叉反应: Human, Mouse, Rat
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暂无相关产品
靶标
基因名
FH
蛋白名
Fumarate hydratase, mitochondrial
亚基
Homotetramer.
亚细胞定位
Cytoplasm, Mitochondrion
组织特异性
Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.
疾病
Fumarase deficiency (FMRD): The disease is caused by mutations affecting the gene represented in this entry. A severe autosomal recessive metabolic disorder characterized by early-onset hypotonia, profound psychomotor retardation, and brain abnormalities, such as agenesis of the corpus callosum, gyral defects, and ventriculomegaly. Many patients show neonatal distress, metabolic acidosis, and/or encephalopathy.
Hereditary leiomyomatosis and renal cell cancer (HLRCC): The disease is caused by mutations affecting the gene represented in this entry. A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients.
Hereditary leiomyomatosis and renal cell cancer (HLRCC): The disease is caused by mutations affecting the gene represented in this entry. A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients.
相似性
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
功能
Also acts as a tumor suppressor.
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